Mouse Tumor Model for Neurofibromatosis Type 1
- 10 December 1999
- journal article
- other
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 286 (5447) , 2176-2179
- https://doi.org/10.1126/science.286.5447.2176
Abstract
Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterized by increased incidence of benign and malignant tumors of neural crest origin. Mutations that activate the protooncogeneras, such as loss ofNf1, cooperate with inactivating mutations at thep53tumor suppressor gene during malignant transformation. One hundred percent of mice harboring nullNf1andp53alleles in cis synergize to develop soft tissue sarcomas between 3 and 7 months of age. These sarcomas exhibit loss of heterozygosity at both gene loci and express phenotypic traits characteristic of neural crest derivatives and human NF1 malignancies.Keywords
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